The Canadian Congenital Diaphragmatic Hernia Collaborative Mobile App: A National Guideline Implementation Strategy.

OBJECTIVE: Coinciding with the publication of the Canadian congenital diaphragmatic hernia (CDH) Collaborative's clinical practice guidelines (CPG), we developed a mobile smartphone app to increase guideline utilization and promote knowledge translation. STUDY DESIGN: This mobile app was organized into sections corresponding to the phases of CDH care (prenatal, perinatal/postnatal, and child/adolescent), and contained 22 recommendations supported by evidence summaries, PubMed links, levels of evidence, and strength of expert consensus. Download statistics were collected from September 2018 to June 2020 after release of two iOS versions and an Android platform. Data regarding user numbers/location, most visited sections, and individual session details were analyzed. RESULTS: During the study period, the CDH app had 1,586 users predominantly from Canada (40%), United States (30%), and Brazil (12%). The Android release increased app visibility, particularly in Brazil, which had the largest number of new users. Of 3,723 sessions, roughly one-third were returning users. The average session duration and screens viewed/session was 4 minutes and seven screens, respectively. Postnatal ventilation was the most frequently visited subsection after prenatal diagnosis/risk stratification. Measurement of observed-to-expected lung head ratio was the most visited individual recommendation. The guideline compliance checklist was the most frequently accessed resource highlighting its utility. CONCLUSION: The CDH app is an innovative platform to disseminate guidelines. The increasing global reach of the app suggests worldwide CPG relevance. With additional features planned, the CDH app will continue to support clinical decision-making and empower patients and families as they navigate the short and long-term challenges associated with CDH. KEY POINTS: · Mobile smartphone technology provides an optimal platform for guideline dissemination.. · International uptake supports worldwide CPG relevance.. · Future initiatives include the development of patient and family resources..

The Canadian Pediatric Surgery Network Congenital Diaphragmatic Hernia Evidence Review Project: Developing national guidelines for care.

The Canadian Pediatric Surgery Network (CAPSNet) has been collecting population-based data regarding congenital diaphragmatic hernia (CDH) across its 17 perinatal sites since 2005. With >500 infants registered to date, CAPSNet has addressed many critical knowledge gaps pertaining to CDH care. Most importantly, it has identified variability in both CDH practice and outcome across Canada. Using the successful Evidence-based Practice for Improving Quality (EPIQ) method, CAPSNet is undertaking a national, multidisciplinary effort to standardize best practices for CDH, from prenatal diagnosis to hospital discharge, based on the best available evidence. The present article outlines the value of clinical research networks and the process CAPSNet will undertake to produce national consensus guidelines for CDH care.

Le Réseau canadien de chirurgie pédiatrique recueille des données en population sur la hernie diaphragmatique congénitale (HDC) dans 17 sites périnatals depuis 2005. Puisque plus de 500 nourrissons y sont inscrits jusqu'à présent, le Réseau a corrigé de nombreuses lacunes liées aux soins de la HDC. Qui plus est, il a décelé la variabilité dans la pratique et les résultats de la HDC au Canada. Selon la méthode EPIC (un acronyme anglais qui signifie pratique fondée sur des données probantes pour améliorer la qualité), le Réseau déploie des efforts nationaux et multidisciplinaires pour normaliser les pratiques exemplaires en matière de soins de la HDC, du diagnostic prénatal au congé de l'hôpital, d'après les meilleures données probantes. Le présent article souligne la valeur des réseaux de recherche clinique et le processus que le Réseau entreprendra pour produire des lignes directrices consensuelles nationales sur les soins de la HDC.

The promise and pitfalls of care standardization in congenital diaphragmatic hernia.

The aim of standardizing care is to enhance patient outcomes and optimize healthcare delivery by minimizing variations in care and ensuring the efficient allocation of healthcare resources. Despite these potential benefits to patients, healthcare providers and the healthcare system, standardization may also disadvantage these groups. With a specific focus on congenital diaphragmatic hernia, this article will review the promise and pitfalls of standardization, as well as a potential path forward that uses standardization to improve outcomes in this rare and complex disease process.

How can pediatric surgeons address racism and become actively anti-racist?

BACKGROUND/PURPOSE: This is an article submitted on behalf of the Canadian Association of Pediatric Surgeons. We assert that Pediatric Surgeons must work to dismantle systemic racism. Pediatric Surgeons have expertise in both common and rare surgical diseases affecting patients ranging from premature neonates to adolescents. Thus, our professional obligation is to transform our health and social systems to prevent the harms of racism to our patients. METHODS: Specific to the Canadian context, we describe a brief history, the ongoing impact on individuals and communities, and the harmful effect on the surgical community and trainees. Finally, we developed a series of practical recommendations to help surgeons become actively anti-racist. RESULTS: Four primary recommendations are made: (1) Increasing and supporting anti-racism education; (2) Changing individual behaviours to combat racism; (3) Developing strategies for organizational change; and (4) Committing to diversity in leadership. CONCLUSION: As surgeons, we are actors of change, and we can take meaningful steps to combat racism in our health systems. LEVEL OF EVIDENCE: V.

The First Two Years of the Association of Pediatric Surgery Training Program Directors (APSTPD) Transition to Fellowship Course: Lessons Learned and Future Directions.

OBJECTIVE: The first transition to fellowship course for incoming pediatric surgery fellows was held in the US in 2018 and the second in 2019. The course aimed to facilitate a successful transition in to fellowship by introduction of the professional, patient care, and technical aspects unique to pediatric surgery training. The purpose of this study was to evaluate the feasibility and effectiveness of the first two years of this course in the US and discuss subsequent evolution of this endeavor. DESIGN: This is a descriptive and qualitative analysis of two years' experience with the Association of Pediatric Surgery Training Program Directors' (APSTPD) Transition to Fellowship course. Course development and curriculum, including clinical knowledge, soft skills, and hands-on skills labs, are presented. Participating incoming fellows completed multiple choice, boards-style pre- and post-tests. Scores were compared to determine if knowledge was effectively transferred. Participants also completed post-course evaluations and subsequent 3- or 12-month surveys inquiring on the lasting impact of the course on their transition into fellowship. Standard univariate statistics were used to present results. SETTING: The first APSTPD Transition to Fellowship course was held at the Johns Hopkins Hospital in Baltimore, Maryland in 2018, and the second course was held at the Oregon Health and Science University in Portland, Oregon in 2019. PARTICIPANTS: All fellows entering ACGME-certified Pediatric Surgery fellowships in the United States were invited to participate. Twenty fellows accepted and attended in 2018, and fourteen fellows participated in 2019. RESULTS: There were 34 incoming pediatric surgery fellow participants over 2 years. Faculty represented more than 10 institutions each year. Pre- and post-test scores were similar between years, with a significant improvement of scores after completion of the course (67±10% vs 79±8%, p < 0.001). Feedback from participants was overwhelmingly positive, with skills labs being attendees' favorite component. When asked about usefulness of individual course sessions, more attendees found clinical sessions more useful than soft skills (93% vs 73%, p = 0.011). Almost all (90%) of participants reported the course met its stated purpose and would recommend the course to future fellows. This was further reflected on 3 and 12 month follow up surveys wherein 85% stated they found the course helpful during the first few months of fellowship and 90% would still recommend it. CONCLUSIONS: A transition to fellowship course in the US for incoming pediatric surgery fellows is logistically feasible, effective in transfer of knowledge, and highly regarded among attendees. Feedback from each course has been used to improve the subsequent courses, ensuring that it remains a valuable addition to pediatric surgical training in the US.

Congenital diaphragmatic hernia.

Congenital diaphragmatic hernia (CDH) is a rare birth defect characterized by incomplete closure of the diaphragm and herniation of fetal abdominal organs into the chest that results in pulmonary hypoplasia, postnatal pulmonary hypertension owing to vascular remodelling and cardiac dysfunction. The high mortality and morbidity rates associated with CDH are directly related to the severity of cardiopulmonary pathophysiology. Although the aetiology remains unknown, CDH has a polygenic origin in approximately one-third of cases. CDH is typically diagnosed with antenatal ultrasonography, which also aids in risk stratification, alongside fetal MRI and echocardiography. At specialized centres, prenatal management includes fetal endoscopic tracheal occlusion, which is a surgical intervention aimed at promoting lung growth in utero. Postnatal management focuses on cardiopulmonary stabilization and, in severe cases, can involve extracorporeal life support. Clinical practice guidelines continue to evolve owing to the rapidly changing landscape of therapeutic options, which include pulmonary hypertension management, ventilation strategies and surgical approaches. Survivors often have long-term, multisystem morbidities, including pulmonary dysfunction, gastroesophageal reflux, musculoskeletal deformities and neurodevelopmental impairment. Emerging research focuses on small RNA species as biomarkers of severity and regenerative medicine approaches to improve fetal lung development.

Risk stratification helps identify congenital diaphragmatic hernia (CDH) infants in need of formal neurodevelopmental assessment: Observations from a structured, interdisciplinary long-term follow-up clinic.

BACKGROUND/PURPOSE: Neurodevelopmental delay (NDD) affects congenital diaphragmatic hernia (CDH) infants. Initial assessment by experienced developmental pediatricians, supported by Bayley-3 tests, is a viable pathway for NDD identification and surveillance. We risk stratified CDH infants to observe differences in incidence and type of NDD based on disease severity. METHODS: Patients from a CDH long-term follow-up database started in 2012 were reviewed (REB#2019-4583). Risk stratification into low, moderate, and high-risk cohorts was performed using the CDH Study Group Mortality Prediction Score. Patients requiring ECLS, supplemental oxygen at 30 days and patch repair were also considered high-risk (i.e. usual clinical criteria). Post-discharge NDD assessments by developmental pediatricians and occupational therapists (Bayley-3) were analyzed for all patients >18months. NDD incidence and type per risk group was determined using descriptive statistics. RESULTS: Of 102 CDH patients included for study, 26% (27/102) had NDD. Risk stratification identified 2(2%), 7(7%), and 18(18%) patients with NDD in the low, moderate and high-risk groups, respectively. Language delay (2 low; 6 moderate; 10 high) was the most prevalent. Three patients had both expressive and receptive language delay. Motor deficits were observed almost exclusively in the high-risk group. CONCLUSION: Based on our experience, NDD affects one-quarter of CDH infants. Risk stratification helped identify infants at increased risk of NDD. While language delays predominated across all risk groups, multiple deficits occurred in higher risk cohorts. These patients should receive structured NDD assessment as part of an optimal interdisciplinary CDH care pathway.

The future is here! Pediatric surgery and the move to the royal college of physicians and surgeons of Canada's competence by design.

This interactive session was held at the 51st Annual Meeting of the Canadian Association of Pediatric Surgeons (CAPS) in preparation for the transition of Pediatric Surgery training in Canada to Competency by Design (a CBME-based model of residency training developed by the Royal College of Physicians and Surgeons of Canada).

Lethal outcome after percutaneous aspiration of a presumed ovarian cyst in a neonate.

Most surgeons agree that complex neonatal ovarian cysts, regardless of size, warrant operative intervention. Management of simple cysts >4 cm is still controversial, although many favor intervention because of the increased risk of torsion. Whereas laparoscopic cyst resection is favored by some, others prefer less invasive percutaneous needle aspiration. We present a newborn infant who was admitted with sepsis and respiratory failure after home delivery. Ultrasound done on day 8 to check for umbilical venous line placement incidentally showed a simple cyst measuring 3.6 x 5.9 x 6.9 cm that was presumed to be of ovarian origin. Percutaneous needle aspiration was atraumatic and revealed serous fluid, with a high estradiol level. Four days later, surgery was indicated for clinical deterioration with suspected hemorrhage into the cyst. We found a midgut volvulus with extensive necrosis secondary to a jejunal duplication cyst. Ovaries were normal and there was no evidence of malrotation. Postoperatively, after discussion with the parents, support was withdrawn and the child died. We should not rely solely on ultrasonographic features and fluid characteristics to diagnose a large neonatal abdominal cyst, but rather confirm the diagnosis with laparoscopy.

Paraplegia after chest wall resection for primitive neuroectodermal tumor.

Spinal cord complications are rarely encountered in elective pediatric surgery. We present a patient who became paraplegic after resection of a chest wall tumor. After neoadjuvant radio- and chemotherapy, a 10-year-old girl with primitive neuroectodermal tumor (PNET) of the right chest underwent a right chest wall resection. Ribs #3, 4, 5, and 6 were resected en masse with a pulmonary wedge resection of right upper and middle lobes. To obtain clear margins, the ribs were disarticulated from the vertebral column. Significant bleeding arose when the fourth rib was detached. Presuming an intercostal vessel bleed, the area was packed with surgicel with resolution of the bleeding. The patient was kept sedated and ventilated in the PICU. The next day, she complained of paresis of her lower extremities. MRI revealed compression of the spinal cord at the T4 level. Emergency decompression and laminectomy was performed. Blood clot and surgicel were found in the area of the spinal canal. Giant epidural veins were encountered as well, again with massive bleeding. The patient never regained neurological function below the insult to the cord. Despite negative margins and ongoing chemo- and radiotherapy, her tumor recurred and she died to her disease less than 1 year later. Chest wall tumors arising near the spinal canal may be associated with enlarged Batson's plexus that may hemorrhage during surgical resection. We recommend immediate imaging or prophylactic laminectomy for any concerns of spinal hemorrhage. Patients should also receive regular assessment of neurovitals, despite issues of pain control and sedation.

The pitfalls of endotracheal intubation beyond the fistula in babies with type C esophageal atresia.

The intraoperative management of a neonate with esophageal atresia (EA) and distal tracheoesophageal fistula (TEF) is a true anesthetic challenge. Pediatric anesthesia textbooks recommend a distal tracheal intubation beyond the fistula and spontaneous ventilation, if possible, until surgical control of the fistula is achieved to minimize gastric distention. A full-term neonate with Trisomy 21 presented with an EATEF and was transferred to the operating theater for repair after appropriate evaluation. After induction of anesthesia, a size 3.0 endotracheal tube was inserted orally with confirmation of its position by good air entry and chest movement bilaterally. After positioning for thoracotomy, the patient desaturated and became bradycardic with abdominal distention. Despite reintubation, gastric needle decompression, and bilateral pleural aspiration to exclude pneumothorax, cardiopulmonary resuscitation was unsuccessful and the child died. Autopsy revealed the endotracheal tube in the trachea with its distal end passing through a large distal TEF. Preoperative bronchoscopy may help the team to assess the size and location of the distal TEF and plan for the best anesthetic strategy. It may also be useful to confirm tube location after endotracheal intubation and intraoperatively in the event of cardiorespiratory instability.

Respiratory disorders in patients with omphalocele.

The respiratory difficulties experienced by infants with omphalocele are being appreciated with greater frequency. These problems represent self-limited difficulties related to omphalocele closure or are the result of severe pulmonary disease including pulmonary hypoplasia and pulmonary hypertension. Infants with giant omphalocele represent a unique group that may experience increased respiratory morbidity which may lead to chronic respiratory problems extending into childhood and adolescence. Importantly, respiratory insufficiency at birth is an independent predictor of mortality for patients with omphalocele. In this review, we will provide a summary of the respiratory difficulties experienced by patients with omphalocele as well as insight into management and surveillance.

Preoperative cardiopulmonary evaluation in specific neonatal surgery.

Preoperative assessment of surgical neonates often relates to issues of prematurity, low birth weight, or associated malformations. This review explores the preoperative cardiopulmonary evaluation in specific newborn surgical populations, the role of echocardiography in congenital diaphragmatic hernia perioperative management, the impact of bronchopulmonary dysplasia in the ex-preterm surgical neonate and a brief discussion on the risk of general anesthesia and specific anesthetic considerations for any surgical neonate. Newborns with congenital anomalies requiring early general surgical intervention should have an assessment for congenital heart disease. In the asymptomatic neonate, a thorough physical exam may be sufficient preoperatively. Neonates born with esophageal atresia or anorectal malformations should have a full evaluation for VACTERL associations. Initial echocardiography in congenital diaphragmatic hernia is used to evaluate anatomy, but there is emerging evidence to suggest the use of echocardiography in the ongoing surveillance of CDH to influence the timing of surgical intervention. Bronchopulmonary dysplasia is present in up to 40% of ex-premature neonates and increases the risk of postoperative apneas and need for ventilatory support. However, all surgical neonates have an increased risk of post-operative apneas, and the need for surgical intervention should be balanced with the risk of general anesthesia.

A transition to discipline curriculum for pediatric surgery trainees: Evaluation of a pediatric surgery boot camp from 2017 to 2018.

INTRODUCTION: Boot camps seek to impart knowledge and skills for individuals entering new roles. We sought to evaluate knowledge, skills, and confidence of in-coming pediatric surgery trainees with a 2.5-day pediatric surgery boot camp. METHODS: A curriculum included key aspects of pediatric surgery delivered during interactive lectures, small group discussions, and simulation. With REB approval, participant demographics were collected. Pre- and posttests assessed knowledge and trainee confidence. Comparative statistics and multivariate analysis of variance (MANOVA) were performed. RESULTS: Between 2017 and 2018, 16 individuals from North American pediatric surgery training programs participated in two boot camps. Ten had North American general surgery training, and eleven had no pediatric surgery exposure ≥1 year prior. All participants expressed increased confidence with course material after boot camp [F(18,11) = 3.137;p < 0.05]. Performance improved significantly (pre- vs. posttests, 47.0% vs. 62.4%; p < 0.05). MANOVA between faculty and trainees demonstrated agreement on the value of individual sessions [F(15,3) = 0.642;p = 0.76]. Neonatal bowel obstruction, gastrostomy tube complications, esophageal atresia, pain management, and informed consent were rated most useful. CONCLUSION: Trainees and teaching faculty considered the boot camp valuable. Trainees demonstrated significant improvements in core knowledge and confidence. The initial pediatric surgery boot camp experience shows promise in facilitating the transition to discipline for new trainees. STUDY TYPE: Prospective treatment study. LEVEL OF EVIDENCE: IV.

Prenatal prediction of survival in congenital diaphragmatic hernia: An audit of postnatal outcomes.

PURPOSE: Effective antenatal counseling in congenital diaphragmatic hernia (CDH) relies on proper measurement of prognostic indices. This quality initiative audited the accuracy of prenatal imaging with postnatal outcomes at two tertiary pediatric referral centers. METHODS: Prenatal lung-head ratio (LHR) and total fetal lung volume (TFLV) for CDH patients treated between 2006 and 2017 were retrieved. Study inclusion required at least one LHR or TFLV measurement between 24 and 32 weeks gestational age. Postnatal outcomes [mortality, extracorporeal life support (ECLS) need, patch repair, persistent pulmonary hypertension, oxygen requirement at 28 days] were abstracted from the Canadian Pediatric Surgery Network (CAPSNet) database and local chart review. Univariate and descriptive analyses were conducted. RESULTS: Eighty-two of 121 eligible CDH patients (68%) were included. Overall mortality, ECLS rates, and patch repair were 33%, 12.5%, and 45%, respectively. Lower LHR values correlated with increased rates of each outcome and persisted despite multiple measurements. Values obtained were higher than those in published schemata. LHR values >45% were most associated with survival, avoidance of ECLS, and primary repair. TFLV values only correlated with mortality and patch repair. CONCLUSIONS: This audit confirms that LHR and TFLV values predict CDH outcomes. However, absolute values obtained require careful interpretation and internal review. LEVEL OF EVIDENCE: IV.

Respiratory infections: pneumonia, lung abscess, and empyema.

Pneumonia is an important clinical problem that affects children of all ages. Although effectively treated on an outpatient basis in the majority of cases, some children with respiratory infections still require hospitalization. This may be particularly true for patients with immunocompromise, for whom the lung represents the most common site of infection. Furthermore, respiratory infections represent a significant source of morbidity and mortality in this patient population. This article focuses on the clinical presentation, etiology, and treatment of childhood pneumonia, with special consideration given to the immunocompromised child. Two specific complications of pneumonia, lung abscess and empyema, are discussed.

Risk stratification and outcome determinants in gastroschisis.

Selection of outcome determinants and risk stratification are necessary to identify patients at higher risk for morbidity and mortality. This facilitates human and material resource allocation and allows for improved family counseling. While several different factors, including prenatal ultrasonographic bowel features, the timing and mode of delivery, and the features of bowel injury have been investigated in gastroschisis, there is still significant debate as to which of these best predicts outcome. This article reviews the different outcome predictors and risk prognostication schemata currently available in the literature to help guide clinicians caring for infants with gastroschisis.

Loop versus divided colostomy for the management of anorectal malformations: a systematic review and meta-analysis.

BACKGROUND: The ideal colostomy type for patients with anorectal malformations (ARM) is undetermined. We performed a systematic review and meta-analysis of short-term complications comparing loop and divided colostomies. METHODS: After review registration (PROSPERO: CRD42016036481), multiple databases were searched for comparative studies without language or date restrictions. Gray literature was sought. Complications investigated included stomal prolapse/hernia/retraction, wound infections, and urinary tract infections (UTIs). Two reviewers independently assessed study eligibility and the quality of included studies. Meta-analysis of selected complications was performed using Revman 5.3, with p<0.05 considered significant. RESULTS: Twenty-six studies were included, and four were multi-institutional. Reporting standards were highly variable. Studies scored between 6 and 9 of possible nine stars on the NOS. Overall, 3866 neonates with ARM were incorporated, in which 2241 loop colostomies and 1994 divided colostomies were reported. Of 10 studies reporting short-term complications, the overall rate was 27%. Meta-analysis demonstrated no significant difference in the incidence of UTIs, (OR: 2.55 [0.76, 8.58], p=0.12), while loop colostomies had a significantly higher prolapse rate (See figure). No publication bias was noted. CONCLUSIONS: A colostomy for patients with an ARM is a source of considerable morbidity. Divided colostomies reduce the risk of subsequent prolapse and may represent the preferred approach. LEVEL OF EVIDENCE: 3A.